{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T04:18:53Z","timestamp":1760242733618},"reference-count":10,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2015,3,4]],"date-time":"2015-03-04T00:00:00Z","timestamp":1425427200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2015,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>\u2010associated thrombotic microangiopathy (<jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>) is a severe complication with a poor prognosis. Recently, it has been reported that complement system dysregulation, such as CFH autoantibodies and deletions <jats:styled-content style=\"fixed-case\">CFH<\/jats:styled-content>\u2010related genes 3 and 1, induced <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>. In addition, C4d\u2010positive renal arterioles are both a good marker of complement system activation and a useful diagnostic tool for <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>. Because dysregulation of the complement system is associated with <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>, the complement system might be a therapeutic target, such as eculizumab, a terminal complement inhibitor. Herein, we describe an eight\u2010yr\u2010old boy who developed <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> accompanied by severe renal dysfunction. His renal specimen showed diffuse C4d deposition in the renal arterioles, which is consistent with <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content>. Although the patient gradually improved without eculizumab, renal arteriolar C4d staining in sample with <jats:styled-content style=\"fixed-case\">TA<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">TMA<\/jats:styled-content> shows the complement system activation and may guide the target therapy using the eculizumab.<\/jats:p>","DOI":"10.1111\/petr.12450","type":"journal-article","created":{"date-parts":[[2015,3,4]],"date-time":"2015-03-04T10:14:54Z","timestamp":1425464094000},"source":"Crossref","is-referenced-by-count":13,"title":["Hematopoietic stem cell transplantation\u2010associated thrombotic microangiopathy accompanied by renal arteriolar C4d deposition"],"prefix":"10.1111","volume":"19","author":[{"given":"Kenichi","family":"Sakamoto","sequence":"first","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshihiko","family":"Imamura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinya","family":"Osone","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayako","family":"Nishimura","sequence":"additional","affiliation":[{"name":"Department of Pathology Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masashi","family":"Nishida","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology and Nephrology Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Ishida","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hajime","family":"Hosoi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Science Kyoto Prefectural University of Medicine Kyoto Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2015,3,4]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-02-321315"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.bmt.1705758"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-05-501445"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e31829807aa"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12267"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3801011"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/TP.0b013e318277b2e2"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-03-564997"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1208981"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbmt.2013.12.565"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpetr.12450","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.12450","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T16:00:48Z","timestamp":1695571248000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.12450"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,3,4]]},"references-count":10,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2015,6]]}},"alternative-id":["10.1111\/petr.12450"],"URL":"https:\/\/doi.org\/10.1111\/petr.12450","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,3,4]]}}}